Primary central nervous system lymphoma pathophysiology

Primary central nervous system lymphoma Microchapters
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Sujit Routray, M.D. [2]

Associated Conditions

Primary central nervous system lymphoma is highly associated with Epstein-Barr virus (EBV) infection (> 90%) in immunodeficient patients (such as those with AIDS and those iatrogenically immunosuppressed).

Gross Pathology

• Primary central nervous system lymphoma presents as a solitary or multiple, well-defined or infiltrating mass lesion/s that can arise in the cortex, white matter, or deep grey matter (more common in low-grade lesions).
• They may demonstrate areas of necrosis, especially in immunodeficient patients.
• Origin of malignant cells is not well understood as intra-axial CNS does not have lymphatic system.

Microscopic Pathology

• The vast majority (>90%) of primary central nervous system lymphoma are B-cell in origin: diffuse large B-cell lymphoma and high-grade Burkitt-like B-cell lymphoma.
• Malignant cells tend to accumulate around blood vessels.
• Low-grade tumors are more frequently T-cell in origin.
• On microscopic histopathological analysis, primary central nervous system lymphoma is characterized by a perivascular distribution composed of large cells with prominent nucleoli.

Gallery

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  _{Micrograph from a brain biopsy demonstrating a primary CNS lymphoma with the characteristic perivascular distribution composed of large cells with prominent nucleoli, on HPS stain.}

References

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